Castleman’s Disease: A Rare Disease & Diagnostic Challenge
A 50-year-old woman presented to the hospital with increasing fatigue as her sole symptom. Diagnostic investigations revealed an interaortocaval, retroperitoneal tumor mass in her upper abdomen as the only manifestation of the disease. No enlarged lymph nodes were detected.

A laparotomy was conducted with radical extirpation of the tumor mass (10×9×5.7cm). Complete tumor resection with clear margins was achieved. A pathological analysis of the resected sample showed atypical lymphoid tissue of small to medium cells with some clearly visible nucleoli, enlarged sinusoidal vessels, pleomorphic calcifications and focally preserved germinal-center-like structures.

Histological and immunohistochemical analysis confirmed the diagnosis of Castleman’s disease: staining for CD3, CD5, CD10, CD20, CD23, CD79 and Ki-67 was strongly positive in the germinal-center-like structures. Histological findings clearly showed the disease to be the hyaline vascular subtype.

Points Worth Remembering :-
1. Castleman’s Disease remains difficult to diagnose preoperatively because the disease may resemble other malignant tumors. Therefore, exclusion of other diseases with Lymphadenopathy, for example Lupus erythematodes, Thymoma, Sarcoma, Rheumatoid arthritis, HIV infection and Lymphoma, is important for differential diagnosis.
2. Also known as Angiofollicular lymph node Hyperplasia, there are two known expansion types of this disease, unicentric and multicentric, which play a major role in determining therapy.
3. Although pathological analysis still remains the gold standard for diagnosis, MRI, contrast-enhanced Sonography, Angiography and CT are valuable diagnostic tools. MRI can be helpful in identifying the hypervascular appearance of the hyaline vascular type.


Source: https://jmedicalcasereports.biomedcentral.com/articles/10.1186/1752-1947-8-93
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